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https://www.readbyqxmd.com/read/29329127/cutis-laxa-acquisita-after-urticarial-vasculitis-in-sle-patients
#1
Kimberly B Golisch, Silvija P Gottesman, Patricia Ferrer, Keliegh S Culpepper
Cutis laxa is a rare connective tissue disease involving damage to dermal elastic fibers creating a clinical appearance of loose, sagging skin. The condition can be either acquired or genetic. Autoimmune diseases, neoplasms, infections, and medications have been proposed as the cause of, or in association with, the acquired form. In nearly 50% of cases, erythematous plaques present before the onset of cutis laxa. Separately, urticarial vasculitis and systemic lupus erythematosus have been linked to cutis laxa acquisita...
January 11, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29328507/genetics-of-immune-mediated-inflammatory-diseases
#2
REVIEW
Trixy David, Stephanie Ling, Anne Barton
Immune-mediated inflammatory diseases (IMIDs) are characterised by dysregulation of the normal immune response, which leads to inflammation. Together, they account for a high disease burden in the population, given that they are usually chronic conditions with associated co-morbidities. Examples include systemic lupus erythematosus, rheumatoid arthritis, Crohn's disease and type 1 diabetes. Since the advent of genome-wide association studies, evidence of considerable genetic overlap in the loci predisposing to a wide range of IMIDs has emerged...
January 12, 2018: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29324237/dna-induction-of-mdm2-promotes-proliferation-of-human-renal-mesangial-cells-and-alters-peripheral-b-cells-subsets-in-pediatric-systemic-lupus-erythematosus
#3
Chen-Xing Zhang, Ji Chen, Li Cai, Jing Wu, Jia-Yuan Wang, Lan-Fang Cao, Wei Zhou, Tong-Xin Chen
The study is aimed to investigate the role of MDM2 in the pathogenesis of lupus nephritis (LN) in pediatric SLE (pSLE). We confirmed that MDM2 expression was increased in peripheral blood mononuclear cells (PBMCs) as well as renal specimen of SLE compared with that of controls by western blot and immunofluorescence staining. Percentage of apoptotic and necrotic CD4+T, CD8+T and B cells were detected by flow cytometry respectively and levels of plasma cell free DNA (cfDNA) were quantified in SLE and healthy controls (HC)...
January 8, 2018: Molecular Immunology
https://www.readbyqxmd.com/read/29317407/a20-haploinsufficiency-ha20-clinical-phenotypes-and-disease-course-of-patients-with-a-newly-recognised-nf-kb-mediated-autoinflammatory-disease
#4
Florence A Aeschlimann, Ezgi D Batu, Scott W Canna, Ellen Go, Ahmet Gül, Patrycja Hoffmann, Helen L Leavis, Seza Ozen, Daniella M Schwartz, Deborah L Stone, Annet van Royen-Kerkof, Daniel L Kastner, Ivona Aksentijevich, Ronald M Laxer
OBJECTIVES: The association between mutations in TNFAIP3, encoding the NF-kB regulatory protein A20, and a new autoinflammatory disease has recently been recognised. This study aims at describing the clinical phenotypes and disease course of patients with A20 haploinsufficiency (HA20). METHODS: Data for all cases from the initial publication, and additional cases identified through collaborations since, were collected using standardised data collection forms. RESULTS: A total of 16 patients (13 female) from seven families with a genetic diagnosis of HA20 were included...
January 9, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29284768/fitz-hugh-curtis-syndrome-caused-by-gonococcal-infection-in-a-patient-with-systemic-lupus-erythematous-a-case-report-and-literature-review
#5
Darío A Rueda, Luisina Aballay, Lisandro Orbea, Diego A Carrozza, Paola Finocchietto, Silvia B Hernandez, Mariano M Volpacchio, Horacio di Fonzo
BACKGROUND Fitz-Hugh-Curtis (FHC) syndrome is a perihepatitis linked to inflammatory pelvic disease. It can be caused by Neisseria gonorrhoeae or Chlamydia trachomatis infections. FHC syndrome usually presents with pain in the right hypochondrium and fever, associated with symptoms and signs of pelvic infection in women. CASE REPORT We present the case of a 22-year-old woman with systemic lupus erythematous (SLE) who presented with polyarthritis, cutaneous lesions, and abdominal pain. The diagnosis of FHC syndrome was based on the findings of abdominal computerized tomography (CT) and the isolation of Neisseria gonorrhoeae (NG) in blood cultures...
December 29, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29284338/catastrophic-antiphospholipid-syndrome-and-pregnancy-clinical-report
#6
J Khizroeva, V Bitsadze, A Makatsariya
We have observed the development of a catastrophic antiphospholipid syndrome (CAPS) in a pregnant woman hospitalized at 28 weeks of gestation with a severe preeclampsia. On the same day, an eclampsia attack developed, and an emergency surgical delivery was performed. On the third day, multiorgan failure developed. Examination showed a persistent circulation of lupus anticoagulant, high level of antibodies to cardiolipin, b2-glycoprotein I, and prothrombin. The usual diagnosis of the severe preeclampsia masked a catastrophic antiphospholipid syndrome, exacerbated by the coincident presence of several types of antiphospholipid antibodies...
December 28, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29275858/engineered-sialylation-of-pathogenic-antibodies-in%C3%A2-vivo-attenuates-autoimmune-disease
#7
Jose D Pagan, Maya Kitaoka, Robert M Anthony
Self-reactive IgGs contribute to the pathology of autoimmune diseases, including systemic lupus erythematosus and rheumatoid arthritis. Paradoxically, IgGs are used to treat inflammatory diseases in the form of high-dose intravenous immunoglobulin (IVIG). Distinct glycoforms on the IgG crystallizable fragment (Fc) dictate these divergent functions. IgG anti-inflammatory activity is attributed to sialylation of the Fc glycan. We therefore sought to convert endogenous IgG to anti-inflammatory mediators in vivo by engineering solubilized glycosyltransferases that attach galactose or sialic acid...
December 11, 2017: Cell
https://www.readbyqxmd.com/read/29275837/plasticity-and-heterogeneity-of-th17-in-immune-mediated-kidney-diseases
#8
REVIEW
Christian F Krebs, Ulf Panzer
Anti-neutrophil cytoplasmatic antibody (ANCA)-associated glomerulonephritis, anti-glomerular basement membrane (GBM) glomerulonephritis and lupus nephritis are the most common causes of rapid progressive glomerulonephritis (RPGN) in the Western world. These aggressive forms of autoimmune kidney diseases significantly contribute to end-stage renal disease and are associated with high morbidity and mortality. Moreover, patients show significant heterogeneity with respect to clinical outcome and response to therapy...
December 21, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29261752/a-comparison-of-health-related-quality-of-life-hrqol-across-four-systemic-autoimmune-rheumatic-diseases-sards
#9
Julia Greenfield, Marie Hudson, Evelyne Vinet, Paul R Fortin, Vivian Bykerk, Christian A Pineau, Mianbo Wang, Sasha Bernatsky, Murray Baron
OBJECTIVES: To compare physical and mental health-related quality of life (HRQoL) across four systemic autoimmune rheumatic diseases (SARD). METHODS: Incident subjects enrolled in four SARD cohorts, namely systemic lupus erythematosus (SLE), systemic sclerosis (SSc), rheumatoid arthritis (RA) and idiopathic inflammatory myopathies (IIM) were studied. The outcomes of interest were baseline Short Form Health Survey physical (PCS) and mental (MCS) component summary scores...
2017: PloS One
https://www.readbyqxmd.com/read/29245299/the-introspection-on-the-diagnosis-and-treatment-process-of-a-case-of-guillain-barr%C3%A3-syndrome-gbs-attributed-to-systemic-lupus-erythematosus-sle-a-case-report
#10
Nan Zhang, Jie Cao, Meng Zhao, Li Sun
RATIONALE: Systemic lupus erythematosus (SLE) is an autoimmune inflammatory connective tissue disorder. It may cause neurologic damage which is mainly characterized by central and mental system, while peripheral sexual damage is relatively rare in which Guillain-Barré Syndrome (GBS) as the first performance is more rare . GBS is an autoimmune peripheral neuropathy usually triggered by an antecedent bacterial or viral infection, with SLE being a rare cause. PATIENT CONCERNS: A 65-year-old male presented to the hospital with progressive numbness and adynamia in extremities...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29234732/endovascular-aneurysm-repair-evar-of-an-infrarenal-abdominal-aortic-aneurysm-aaa-in-a-young-patient-with-systemic-lupus-erythematosus-sle
#11
Nick Z P Ng, Tze Tec Chong
Introduction: While hypertension, pericardial, myocardial, and coronary artery disease are common cardiovascular manifestations of systemic lupus erythematosus (SLE), aortic aneurysms (AA) are rare but increasingly diagnosed, with the true incidence unknown. Case report: A 40 year old female suffering from SLE with a 5.3 cm saccular eccentric infrarenal abdominal aortic aneurysm (AAA) was treated successfully with endovascular aneurysm repair (EVAR) using the Medtronic Endurant II bifurcated stent graft and followed up 2 years post-operatively...
2017: EJVES Short Reports
https://www.readbyqxmd.com/read/29226052/developing-a-continuous-quality-improvement-assessment-using-a-patient-centered-approach-in-optimizing-systemic-lupus-erythematosus-disease-control
#12
Katelyn Mariko Updyke, Brittany Urso, Shazia Beg, James Solomon
Systemic lupus erythematosus (SLE) is a multi-organ, autoimmune disease in which patients lose self-tolerance and develop immune complexes which deposit systemically causing multi-organ damage and inflammation. Patients often experience unpredictable flares of symptoms with poorly identified triggers. Literature suggests exogenous exposures may contribute to flares in symptoms. An online pilot survey was marketed globally through social media to self-reported SLE patients with the goal to identify specific subpopulations who are susceptible to disease state changes based on analyzed exogenous factors...
October 9, 2017: Curēus
https://www.readbyqxmd.com/read/29208331/autoimmune-and-medication-induced-lymphadenopathies
#13
REVIEW
Alejandro A Gru, Dennis P O'Malley, Aliso Viejo
This article will provide a discussion of some common autoimmune disorders that could affect the lymph nodes and potentially mimic B and T-cell lymphomas. Some of these disorders are more characteristic of individuals in the pediatric age group (autoimmune lymphoproliferative syndrome, Kawasaki disease), while others present in older individuals (rheumatoid arthritis, lupus erythematosus, sarcoidosis). A common finding that groups all of these disorders together is the overall relative preservation of the architecture, a feature that can be particularly helpful to distinguish them from many B and T-cell lymphomas...
November 28, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29179962/the-spectrum-of-glomerular-disease-between-the-years-2003-and-2015-in-columbia-a-review-of-12-613-cases
#14
Luis Eduardo Barrera-Herrera, Rocío Del Pilar López Panqueva, Adriana Alejandra Flórez Vargas, Rafael Enrique Andrade Pérez
BACKGROUND: The prevalence of glomerular disease (GD) varies according to the different socio-demographic characteristics of each population. For the first time we present the prevalence of the different forms of GD among patients from several different areas of Columbia. METHODS: Data from 12,613 renal biopsies studied at our University Hospital between 2003 and 2015 was reviewed. Pathology results were classified according to a list of renal diseases proposed by various authors...
January 2017: Revista Española de Patología
https://www.readbyqxmd.com/read/29178378/prevalence-of-arthritis-revisited
#15
EDITORIAL
Jeffrey N Katz
Estimates of disease prevalence have far-reaching consequences. By providing a baseline understanding of a disease's impact, these estimates are used to justify investment in research, prevention and treatment. For years, estimates of arthritis prevalence have been derived from a single screening question on the National Health Interview Survey (NHIS): "Have you ever been told by a doctor or other health professional that you have some form of arthritis, rheumatoid arthritis, gout, lupus, or fibromyalgia?" This question alone grounds the prevailing estimates of about 54 million US adults living with arthritis...
November 27, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29168029/the-chinese-version-of-the-sleqol-is-a-reliable-assessment-of-health-related-quality-of-life-in-han-chinese-patients-with-systemic-lupus-erythematosus
#16
Hai-Zhi Jiang, Zhi-Guo Lin, Hong-Juan Li, Qing -Du, Wei -Tian, Shu-Ya Wang, Shang-Qi Guan, Yi-Fang Mei
To assess the health-related quality of life (HRQOL) of Han Chinese people with systemic lupus erythematosus (SLE) using a Chinese version of the Systemic Lupus Erythematosus-Specific Quality of Life Questionnaire (SLEQOL-C) and explore the factors influencing HRQOL of people with SLE. Participants were Han Chinese people with SLE. The SLEQOL-C and 36-item Short Form Health Survey (SF-36) were used to estimate the HRQOL. Disease activity was determined using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and fatigue using the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F)...
November 22, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29166758/clinical-significance-of-anti-dense-fine-speckled-70-antibody-in-patients-with-fibromyalgia
#17
Jisoo Jeong, Dong Hyun Kim, Gun Park, Suyeon Park, Hyun-Sook Kim
Background/Aims: Fibromyalgia (FM) is a common rheumatologic disease characterized by chronic widespread pain, along with various clinical manifestations including atypical autoimmune characteristics. Despite its high prevalence, there remain no approved laboratory tests to identify specific manifestations of FM, or to rule out FM from other rheumatic diseases. Anti-dense fine speckled 70 (anti-DFS70) antibodies were initially identified as a form of anti-nuclear antibodies in a patient with interstitial cystitis...
November 24, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29161471/peer-to-peer-mentoring-for-african-american-women-with-lupus-a-feasibility-pilot
#18
Edith M Williams, J Madison Hyer, Ramakrishnan Viswanathan, Trevor D Faith, Delia Voronca, Mulugeta Gebregzaibher, Jim C Oates, Leonard Egede
OBJECTIVE: To examine the feasibility and potential benefits of peer mentoring to improve the disease self-management and quality of life of individuals with systemic lupus erythematosus (SLE). METHODS: Peer mentors were trained and paired with up to three mentees to receive self-management education and support by telephone over 12 weeks. This study took place at an academic teaching hospital in Charleston, South Carolina. Seven quads consisting of one peer mentor and three mentees were matched based on factors such as age, area of residence, marital and work status...
November 21, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29158554/genomic-consequences-of-intensive-inbreeding-in-an-isolated-wolf-population
#19
Marty Kardos, Mikael Åkesson, Toby Fountain, Øystein Flagstad, Olof Liberg, Pall Olason, Håkan Sand, Petter Wabakken, Camilla Wikenros, Hans Ellegren
Inbreeding (mating between relatives) is a major concern for conservation as it decreases individual fitness and can increase the risk of population extinction. We used whole-genome resequencing of 97 grey wolves (Canis lupus) from the highly inbred Scandinavian wolf population to identify 'identical-by-descent' (IBD) chromosome segments as runs of homozygosity (ROH). This gave the high resolution required to precisely measure realized inbreeding as the IBD fraction of the genome in ROH (F ROH). We found a striking pattern of complete or near-complete homozygosity of entire chromosomes in many individuals...
November 20, 2017: Nature Ecology & Evolution
https://www.readbyqxmd.com/read/29152588/the-delivery-science-rapid-analysis-program-a-research-and-operational-partnership-at-kaiser-permanente-northern-california
#20
Julie A Schmittdiel, Rick Dlott, Joseph D Young, Michael B Rothmann, Wendy Dyer, Alyce S Adams
Introduction: Health care researchers and delivery system leaders share a common mission to improve health care quality and outcomes. However, differing timelines, incentives, and priorities are often a barrier to research and operational partnerships. In addition, few funding mechanisms exist to generate and solicit analytic questions that are of interest to both research and to operations within health care settings, and provide rapid results that can be used to improve practice and outcomes...
October 2017: Learning Health Systems
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